Erythrokeratoderma Variabilis Successfully Treated with Topical Tazarotene

Abstract

Erythrokeratoderma variabilis, also known as Mendes da Costa syndrome, is a genodermatosis belonging to the group of diseases known as the erythrokeratodermias. Erythrokeratoderma variabilis is characterized by two distinctive manifestations: well-demarcated, variable, transient, figurate patches of erythema, and localized or generalized hyperkeratotic plaques. Treatments include topical retinoic acid, salicylic acid, and alpha-hydroxy acid in petrolatum, but all have been reported to have limited, variable success rates. We report a child with erythrokeratoderma variabilis with no family history of this entity, successfully treated with topical tazarotene.

Erythrokeratodermia variabilis: Two case reports

“…The first-line treatments of PSEK include topical keratolitics, topical retinoids, tazarotene, alpha-hydroxy acid, and topical corticosteroid. [121213141516] However, some experts suggest systemic retinoids instead of topical therapies. [16] Second-line treatments are etretinate, isotretinoin, and acitretin.…”

Overview of skin diseases linked to connexin gene mutations

“…As the name suggests, it is characterized by coexistent fixed hyperkeratotic plaques and transient erythematous patches that vary in size, shape, and location. Often one of the features may be more dominant than the other, or one may be completely absent . There are two clinical subtypes of EKV.…”

“…The first type has generalized, persistent, dark brown‐gray hyperkeratosis with accentuated skin markings, fine attached scale, and rarely ichthyosis hystrix‐like appearance, while the second type has localized involvement with fixed, symmetrically distributed, and sharply demarcated brown‐yellow plaques with geographic borders involving the buttocks, trunk, extensor surfaces, and extremities (Fig. a–c, f and h) . Hyperkeratotic plaques may have collarette‐like peeling and verrucous surface.…”

“…Hyperkeratotic plaques may have collarette‐like peeling and verrucous surface. The face and scalp are usually spared …”

“…Erythematous patches usually appear first at about six months old and are characterized by their variability. They are well demarcated but can vary in size, shape, location, and duration . Patches may be annular, polycyclic, or geographic in appearance and may last a few hours to days (Fig.…”